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重症肌无力:一家机构收治的104例患者的临床表现与转归

Myasthenia gravis: presentation and outcome in 104 patients managed in a single institution.

作者信息

Al-Moallem Mansour A, Alkali Nura H, Hakami Mohammed A, Zaidan Radwan M

机构信息

Department of Medicine, King Khaled University Hospital, King Saud University, Riyadh, Saudi Arabia.

出版信息

Ann Saudi Med. 2008 Sep-Oct;28(5):341-5. doi: 10.5144/0256-4947.2008.341.

Abstract

BACKGROUND AND OBJECTIVES

Few studies have attempted to delineate the clinical profile of myasthenia gravis (MG) among people of Arab ancestry. Therefore, we sought to clarify the clinical profile, the outcome of treatment and the role of thymectomy in non-thymomatous MG in Saudi Arabia.

PATIENTS AND METHODS

We retrospectively studied 104 patients followed over a mean period of 7.2 years (range, 1 to 22 years) at the King Khaled University Hospital, Riyadh, Saudi Arabia. Disease outcomes were compared among thymectomized and non-thymectomized patients according to the post-intervention status criteria of the Myasthenia Gravis Foundation of America (MGFA).

RESULTS

Age of onset was 22.5+/-9.3 years (meanA+/-SD) in females and 28.2+/-15.9 years in males, with peaks in the second and third decades among females and the third and fourth decades among males. At diagnosis, a majority of patients had moderate generalized weakness, equivalent to MGFA class III severity. After medical treatment with or without thymectomy, 9.6% of all patients had achieved complete stable remission, 3.8% had pharmacological remission, 27.9% had minimal manifestations, 23.1% were improved, 20.2% were unchanged and 15.4% were worse. Only thymectomized patients without a thymoma achieved remission, a significant benefit over those who had no thymectomy (P=.02).

CONCLUSION

MG presents at a younger age among Saudi Arabs compared to other racial groups. Thymectomy conferred significant benefits towards achievement of remission.

摘要

背景与目的

很少有研究试图描绘阿拉伯血统人群中重症肌无力(MG)的临床特征。因此,我们旨在阐明沙特阿拉伯非胸腺瘤性MG的临床特征、治疗结果以及胸腺切除术的作用。

患者与方法

我们回顾性研究了沙特阿拉伯利雅得国王哈立德大学医院随访的104例患者,平均随访时间为7.2年(范围1至22年)。根据美国重症肌无力基金会(MGFA)的干预后状态标准,对接受胸腺切除术和未接受胸腺切除术的患者的疾病结果进行比较。

结果

女性发病年龄为22.5±9.3岁(平均±标准差),男性为28.2±15.9岁,女性在第二和第三个十年达到发病高峰,男性在第三和第四个十年达到发病高峰。在诊断时,大多数患者有中度全身无力,相当于MGFA III级严重程度。在接受或未接受胸腺切除术的药物治疗后,所有患者中有9.6%实现了完全稳定缓解,3.8%实现了药物缓解,27.9%有轻微表现,23.1%有所改善,20.2%无变化,15.4%病情恶化。只有无胸腺瘤的胸腺切除患者实现了缓解,比未进行胸腺切除术的患者有显著益处(P = 0.02)。

结论

与其他种族群体相比,沙特阿拉伯人患MG的年龄较轻。胸腺切除术对实现缓解有显著益处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5655/6074486/4c97a8dc16f1/asm-5-341f1.jpg

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